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J Thorac Cardiovasc Surg 2005;129:1457-1458
© 2005 The American Association for Thoracic Surgery

Brief Communication

Esophagectomy and splenectomy in a patient with osteopetrosis

Samantha K. Hendren, MDa,*, Jessica Wang, MDa, Joseph Gorman, MDa, Thomas Peacock, MDc, Diane M. Hershock, MD, PhDb, Ernest F. Rosato, MDa

a Department of Surgery Hospital of the University of Pennsylvania, Philadelphia, Pa
b Department of Hematology, Hospital of the University of Pennsylvania, Philadelphia, Pa
c Easton Hospital, Easton, Pa

Received for publication September 29, 2004; revisions received October 30, 2004; accepted for publication November 4, 2004.

* Address for reprints: Samantha Hendren, MD, Mount Sinai Hospital, Department of Surgery, 600 University Ave, Suite 451, Toronto, Ontario M5G 2J8, Canada (Email: shendren@mtsinai.on.ca).

The first 20% of the full text of this article appears below.

Osteopetrosis is a rare genetic disorder characterized by a failure of osteoclast-mediated bone resorption and by hematologic abnormalities. A patient with osteopetrosis presenting with a distal esophagus cancer and secondary hypersplenism was surgically treated.

Clinical Summary

The patient is a 57-year-old man with osteopetrosis since childhood, complicated by multiple orthopedic fractures (Figure 1), osteomyelitis, splenomegaly, thrombocytopenia, and chronic anemia requiring routine blood transfusions. Several months before diagnosis, he was noted to have an increasing transfusion requirement. The hemoglobin fell to 2.6 g/dL, and upper endoscopy demonstrated bleeding from an adenocarcinoma at the gastroesophageal junction (Figure 2).


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  		Figure 1. Preoperative chest x-ray film reveals the dense skeleton of osteopetrosis and a chronic left humeral fracture.

 

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  		Figure 2. Abdominal computed tomographic scan showing esophageal lesion and splenomegaly. S, Spleen; T, tumor.

 
An exploratory procedure was performed through a left thoracoabdominal incision. The diaphragm was divided circumferentially. A splenectomy was performed with a subsequent intraoperative platelet transfusion. The esophagus was . . . [Full Text of this Article]






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